Temporal Bone Cancer

This volume comprehensively reviews the current literature on temporal bone cancer and the multidisciplinary approaches used to managing these rare tumors. The text will review important medical issues as they specifically relate to  temporal bone cancer such as advanced imaging, pathologic classification, skull base surgery, plastic reconstructive surgery, and advances in osseointegrated implants for hearing restoration. Additional chapters are dedicated to the evaluation and management, diagnostic radiology, surgical planning and techniques, radiotherapy, chemotherapy, and rehabilitation. An emphasis is placed on the multidisciplinary approach required for the optimal care of these rare tumors.

Written by leaders in the field, Temporal Bone Cancer will be an invaluable resource for residents and fellows in Otolaryngology, Neurosurgery and Neurotology, and clinicians with interest in the primary tumors of the temporal bone.

• Language: English
• Publisher: Springer
• Release date: May 3, 2018
• ISBN: 9783319745398

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© Springer International Publishing AG, part of Springer Nature 2018

Paul W. Gidley and Franco DeMonte (eds.)Temporal Bone Cancerhttps://doi.org/10.1007/978-3-319-74539-8_1

1. Overview and Historical Developments

Paul W. Gidley¹  

(1)

Department of Head and Neck Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA

Paul W. Gidley

Email: [e-mail]

Keywords

Ear canal cancerMiddle ear cancerPeriauricular skin cancerParotid cancerTemporal bone cancerSquamous cell carcinomaAdenoid cystic carcinomaCarcinoid tumorMultidisciplinary careHistory of medicine

Overview

The time has come for a book dedicated to the evaluation and management of temporal bone cancer. A rich literature on temporal bone cancer has accumulated over the years. A simple PubMed search using temporal bone cancer reveals more than 5000 articles, with more than 2700 published in the last 20 years. The outstanding articles in this body of literature are cited throughout the chapters in this book.

The main impetus to writing this book has been to encapsulate the learning and experience gained on managing temporal bone cancer. While cancers that involve the temporal bone are very rare, many of the recommendations in this textbook are based on the experience gained from managing over 400 patients with temporal bone cancer. These patients present with a wide array of tumor burden, from very small to prodigious primary tumors. Our approach to management utilizes a comprehensive, multidisciplinary team that includes otology-neurotology, head and neck surgery, neurosurgery, plastic and reconstructive surgery, oculoplastic surgery, anaplastology, audiology, medical oncology, and radiotherapy. These patients are evaluated by team members, and a comprehensive treatment plan is discussed and agreed upon at our weekly Tumor Board.

It is important to recognize that temporal bone cancer covers a wide range of tumor types and tumor locations. While squamous cell carcinoma (SCC) is the most common tumor to involve the temporal bone, many different primary tumor types can occur within the temporal bone. Squamous cell carcinoma is remarkable since it can be found as a primary tumor in the external auditory canal, middle ear, and mastoid. Other tumor types tend to have a more specific site of origin. For instance, adenoid cystic carcinoma occurs primarily in the ear canal since it arises from cerumen glands. Carcinoid tumors usually arise within the middle ear. Endolymphatic sac tumors (ELSTs) arise from the endolymphatic sac; and, as such, they destroy the posterior portion of the temporal bone.

These tumor types have vastly different behaviors. SCC has a propensity toward aggressive invasion and the potential for metastatic spread to parotid and cervical lymph nodes. Recurrence can occur within 2 years, and death quickly follows. In comparison, adenoid cystic carcinoma of the ear canal has proclivity for perineural spread and a tendency for delayed, distant metastasis. Recurrences can occur 10–15 years after primary tumor resection and are usually in the lungs. Both carcinoid tumors and ELSTs tend to recur locally if not completely excised, and their metastatic potential is very low. Clearly these tumor types have very different natural histories and behaviors should not be lumped together. Many chapters are dedicated to separate tumor pathologies in order to explore the natural histories of various histologic types.

Primary tumors of the temporal bone are rare. The incidence is perhaps one to two cases/million population/year. Skin cancer is very common, with an incidence of about 2800 cases/million population/year, with 75% of these occurring within the head and neck. The position and outstanding nature of the external ear makes it vulnerable to damaging rays from the sun. The temporal bone, especially the ear canal, is more likely to be involved secondarily by primary tumors of the periauricular skin and parotid gland (Fig. 1.1) [1].

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Fig. 1.1

Location of temporal bone primary tumors from the Department of Head and Neck Surgery at MD Anderson Cancer Center (used with permission from Gidley PW, DeMonte F. Temporal bone malignancies. Neurosurgery clinics of North America. 2013;24(1):97–110)

The disease under the moniker temporal bone cancer seems to be changing. In the twentieth century, many case reports and case series showed high numbers of patients with chronic otitis media or chronic otorrhea as a precursor to squamous cell carcinoma of the ear canal, middle ear, or mastoid. In the setting of chronic infection, previous mastoidectomy patients developed large, fungating tumors that quickly spread throughout the temporal bone without the restriction of the ear canal wall. These patients seem to be less frequently encountered today in the USA than was previously reported. The reasons for this change are unclear. Perhaps the management of chronic otitis media has improved, and the persistent draining ear is much less common. Alternatively, as skin cancer rates have risen, more skin cancers occur which grow to involve the ear canal or temporal bone. Nonetheless, the percentage of temporal bone cancer patients with a history of chronic otitis media is decreasing.

The ear canal, external ear, mastoid and middle ear, parotid, periauricular skin, and skull base are all potential sites of primary tumors that involve the temporal bone. Some very large tumors involve the temporal bone, making it difficult to determine the site of origin. Establishing the site of origin is important since the overall survival varies widely based on tumor location (Fig. 1.2) [2].

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Fig. 1.2

Overall survival (used with permission from Gidley PW, Thompson CR, Roberts DB, et al. The oncology of otology Laryngoscope 2012;122(2):393–400) [2]

Furthermore, staging systems are based on primary tumor location and extent. The staging of temporal bone cancer is discussed in Chap. 2. The staging system for skin cancer differs from the Pittsburgh staging system for temporal bone cancer in multiple ways (Chap. 6). An external ear cancer is properly staged using the AJCC system for skin cancer, while an external auditory canal cancer is properly staged using the Pittsburgh staging system. Melanoma and sarcoma have vastly different staging systems. Proper staging helps in treatment selection, allows appropriate comparisons on disease burden, and permits projections about prognosis.

As outlined in the History section of this chapter, many different surgical approaches have been developed to manage temporal bone cancer. The salient feature of surgical management is obtaining tumor-free margins. The very definition of unresectable is a final positive margin. Many factors contribute to unresectability in temporal bone cancer surgery. First, bone cannot be examined using frozen section technique. Second, resecting an important structure, such as the internal carotid artery, to obtain a negative margin might result in significant disability. Third, disease may have spread outside the temporal bone, such as to the lower cranial nerves or paraspinal muscles. Lastly, the anatomy of the temporal bone can make surgery within it difficult.

Management strategies and survival outcomes have greatly improved over the last century. For SCC and other tumors confined to the ear canal, the lateral temporal bone resection accomplishes an en bloc, no-touch, tumor-free margin and is associated with high rates of overall survival. The reason seems to be obvious, in that the tumor is confined by the bony canal. Santorini and Hyrtl’s fissures are preformed pathways that facilitate spread to the parotid gland, the temporomandibular joint, or infratemporal fossa. These anatomic pathways permit spread of disease outside of the ear canal, and they contribute to shorter overall survival.

Much lower overall survival rates are reported for tumors that involve the middle ear or mastoid. In analogous terms, the eardrum can be considered the Ohngren’s line for temporal bone cancer. In maxillary sinus cancers, Ohngren’s line divides the sinus into an inferior-anterior compartment that has a favorable prognosis and a posterior-superior compartment that has a poor prognosis [3]. For the temporal bone, tumors that are contained within the ear canal have a more favorable prognosis than tumors that have spread from the ear canal to involve the middle ear.

Once tumors have invaded the middle ear, the air cell system allows unimpeded spread of malignant disease. The mastoid air cell system, the Eustachian tube, the jugular foramen, the carotid canal, and natural dehiscences in the tegmen each allow the spread of tumor to sites outside of the temporal bone. For this reason, tumors of the middle ear, mastoid, and other parts of the temporal bone are much harder to control surgically than tumors confined to the ear canal.

Several chapters in this book are dedicated to the surgical approaches to tumors in various parts of the temporal bone. Each surgical approach has unique challenges; however, the basic tenet remains the same: achieving a negative margin. The pursuit of a negative margin has led to wider and more extensive surgical resections, often entailing large soft tissue and bony defects.

Large soft tissue defects require proper reconstruction. Perhaps one of the greatest advances for the management of head and neck cancer has been the development and refinement of microvascular free tissue transfer. The role of free flaps is explored in the chapter on reconstruction of temporal bone defects. Facial paralysis is common in temporal bone cancer, and rehabilitation of the paralyzed face and care for the paralyzed eyelids are discussed in detail.

History has shown that surgery alone is not sufficient management for most temporal bone cancers beyond T2 stage tumors. Radiotherapy has been used as a definitive treatment and adjuvant treatment for temporal bone cancers since the birth of this medical field. The subsequent history section outlines some of the early pioneers of this modality. The efficacy of radiotherapy for primary temporal bone cancers has been established in its role as adjuvant, postoperative therapy. Multidrug chemotherapy protocols have been developed for the management of advanced head and neck cancer. A role for chemotherapy is emerging in the treatment of temporal bone cancer.

As part of the primary treatment plan, consideration must be given to hearing and cosmetic rehabilitation. Prosthetic rehabilitation of hearing can be through the use of osseointegrated implants and cochlear stimulators . These implants and speech processors replace the sound conducting mechanism of the ear canal and ossicular chain and help to restore hearing in those patients with an intact cochlea. Anaplastologists create extremely life-like prosthetic external ears which can be attached with either osseointegrated implants or adhesives to give patients an improved body image.

History of Temporal Bone Cancer

It has been 100 years since the first case series of temporal bone cancer was assembled by Newhart in 1907 [4]. Over these years, a significant body of literature has developed regarding cancers that affect the temporal bone. Observations have been made regarding the clinical presentation, natural history, histopathology, treatment, and outcomes for these tumors. Many different diagnostic techniques, staging systems, and treatment strategies have been proposed along the way. The authors who contributed to this body of knowledge were leaders in the field of otolaryngology, plastic surgery, radiotherapy, and neurosurgery. This section strives to provide the first comprehensive review on the subject of the history of temporal bone cancer treatment.

Developing the Diagnosis

In the nineteenth century, the field of otology was full of incompetents and charlatans [5]. Many treatments, elixirs, salves, and surgical procedures were proposed to treat hearing loss, tinnitus, and chronic ear infections. Few of these treatments produced beneficial results. Perhaps the only ear complaint that could be alleviated at the time was cerumen impaction .

In the middle of the nineteenth century, several individuals in Ireland, England, Austria-Hungary, France, and Germany began to dedicate themselves to the study of ear maladies. The first description of squamous cell carcinoma of the temporal bone was independently described by Drs. William Wilde and Hermann Schwartze in 1875 [6]. This date is erroneously cited as 1775 by Furstenberg [7] and repeated by subsequent authors [8, 9].

William Wilde (1815–1876) was the son of a physician and grew up in Roscommon in Ireland (Fig. 1.3). He learned medicine and surgery as an apprentice to Abraham Colles (1773–1843; known for Colles’ fracture) in 1832. He also studied at Park Street Medical School of Trinity College under Robert James Graves (1796–1853; known for Graves’ disease) and William Stokes (1804–1878; known for Stokes-Adams syndrome and Cheyne-Stokes respirations). As a young medical student, he saved a child’s life by performing a tracheostomy [10].

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Fig. 1.3

William Wilde (public domain, https://​commons.​wikimedia.​org/​wiki/​File%3AWilliam_​Wilde_​young.​jpg)

During his last year of medical school, he contracted a severe fever; and Graves arranged for him to convalesce as a private physician to a wealthy patient aboard his private yacht, sailing to the Holy Lands. Upon return to Ireland, Wilde published his account of this adventure and the archeologic findings in a two-volume set, which brought him financial success and fame throughout Ireland [11]. Shortly after this, the first of his three illegitimate children, Henry Wilson (1838–1877), was born. Wilson went on to become a famous Irish ophthalmologist in his own right. This book’s success allowed Wilde to study ear and eye disorders in Vienna.

Wilde returned to Ireland and dedicated his early career to tending to the lower classes; news of his talent spread to the upper classes through their servants and maids [12]. Wilde’s work elevated otology to clinical and scientific respectability [5]. He founded St. Mark’s Hospital in 1844, which was the forerunner to the Royal Victoria Eye and Ear Hospital. He edited the Dublin Quarterly Journal of Medical Science, now called The Irish Journal of Medical Science. He wrote an early textbook on ear surgery in 1853 [13].

During the potato famine of 1845–1849, Wilde was appointed the census commissioner and recorded the progress of the famine in minute detail. He showed great ability in organizing and presenting statistical data, and he added questions about the deaf and dumb, which led him to be the first to identify inherited forms of hearing loss. He was later knighted for this statistical and census work in 1864 [14].

He is known for Wilde’s reflex (the light reflex) and Wilde’s incision (the postauricular incision) for mastoid suppuration. However, his son, Oscar Wilde, the playwright and poet, probably has greater name recognition in today’s era. Ironically, Oscar Wilde died of meningoencephalitis secondary to chronic otitis media [15].

Hermann Schwartze (1837–1910) was born in Neuhof, Germany, the son of a gentleman farmer (Fig. 1.4). He studied medicine in Wurzburg and Berlin, receiving his medical degree in 1853 from the University of Berlin. He studied ear disease under Anton von Tröltsch (1829–1890), known for the pouches of von Tröltsch in the middle ear. Schwartze settled in Halle and was professor of otology at the University of Halle. He published several textbooks on ear anatomy and pathology [16–18]. He described simple mastoidectomy using hammer and gouge (Schwartze mastoidectomy ) in 1873, and his technique spread throughout the world as a treatment for chronic otitis media [19]. He is best remembered for his eponymous sign, a reddish discoloration behind the tympanic membrane seen in otosclerosis. In 1864, Schwartze, von Tröltsch, and Adam Politzer founded the Archiv für Ohrenheilkunde, the first journal dedicated to ear disorders. In the last years of his life, Schwartze suffered from a nervous condition of restlessness, vertigo, and delusions. He died of heart failure at age 73. The Hermann-Schwartze medal at the University of Halle is named in his honor.

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Fig. 1.4

Hermann Schwartze (public domain, https://​commons.​wikimedia.​org/​wiki/​File%3AHermann_​Schwartze.​jpg)

The first description of cancer involving the temporal bone with pathologic confirmation is attributed to Adam Politzer in 1883 [20]. Politzer (1835–1920) was a world famous Viennese physician and surgeon and is considered the founder of otology (Fig. 1.5). He developed many techniques and instruments for otologic examination and surgery. His book, A Textbook of Diseases of the Ear, became a standard treatise on otologic conditions and was translated into many languages. In his textbook, he gave an accurate description of cancer in the ear and temporal bone. He reported three cases; the first began in the outer ear and spread to include the ear canal and middle ear and progressed to the dura until death ensued. He reported a second tumor that began within the ear canal and spread to include the frontal bone, sphenoid wing, and inner ear and produced ipsilateral blindness, facial paralysis, and palate weakness. The third case began on the outer ear and extended to the ear canal. Histologically, this tumor invaded the bony spaces of the temporal bone and led him to conclude that surgery is not successful for treating this late-stage disease.

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Fig. 1.5

Adam Politzer (public domain, https://​commons.​wikimedia.​org/​wiki/​File%3ARudolf_​Krziwanek_​-_​Adam_​Politzer.​jpg)

The great otologist and audiologist, Friedrich Bezold (1842–1908), at the University of Munich, only encountered four cases of ear carcinoma in his 24 years [21]. Bezold is best remembered for his eponymous abscess caused by suppurative breakdown of the digastric ridge leading to neck abscess. Kretschmann complied 4 of his own cases of temporal bone cancer from private practice with 12 cases from the literature in 1887 [22]. Zeroni produced an exhaustive review of the literature from 1804 to 1899 and found 121 ear and temporal bone tumors, including 2 of his own cases [23]; however, the primary source could not be found.

The first large literature review published in the American literature was by Dr. Horace Newhart (1872–1945) of Minneapolis, Minnesota (Fig. 1.6). He cataloged and described 34 cases in the literature, and he noted that 85% had chronically discharging ears [4]. Newhart obtained his medical degree at the University of Michigan (1898) and performed postgraduate training in Vienna. He took three trips to Vienna, spending about 3 years in total there. It is unclear if he studied with Politzer. He became Director of the Division of Otolaryngology at University of Minnesota [24]. He wrote several articles on the ear and hearing, especially on testing hearing in children and the use of hearing aids. His article on carcinoma of the middle ear is his most highly cited paper. He served as president of the American Otological Society in 1939–1940.

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Fig. 1.6

Horace Newhart (photo reprinted with permission) [24]

Ceruminoma was first described by Rud Haug, from the Universitäts-Ohrenpoliklinik, Munich, Germany, in 1894 [25]. Haug wrote 11 papers, and this one on ceruminoma was his most highly cited paper. Unfortunately many tumors were classified under this moniker, thus creating confusion for years about the natural history and behavior of ear canal tumors [25]. Given the confusion attached to this term, it was later rendered obsolete by the World Health Organization in 1991.

In 1898, Leopold Treitel of Berlin described the first case of adenocarcinoma of the middle ear [26]. In his paper he describes a 77-year-old woman with a history of light-headedness and dizziness. She had a tumor involving the middle ear and mastoid. The hand-drawn depiction of the microscopic findings shows a clear papillary pattern to this tumor. Nearly, 100 years later, in 1989, Dennis K. Heffner, Armed Forces Institute of Pathology, proposed that papillary adenocarcinoma of the petrous bone is derived from the endolymphatic sac [27]. His paper on lower-grade papillary adenocarcinoma of the endolymphatic sac is his most highly cited paper.

Piecemeal resection of temporal bone cancers was first reported by Hermann Heyer of Worms, Germany, in 1899 [28]. His paper describes how he excised this tumor under chloroform anesthesia. A wide resection involving the zygoma, parotid, sigmoid sinus, and temporal bone down to the cochlea was accomplished. The dura remained intact. This procedure was performed without the use of an operating microscope, high-speed drill, or electrocautery. Hemostasis was achieved with an iodoform-soaked gauze. The resulting wound was allowed to heal by secondary intention with granulation tissue, and the patient was discharged from hospital 1 month later. The patient apparently returned back to work until his tumor recurred on the dura at the margin of the excision; and he died about 1 year after the operation. Heyer advocated such an operation as palliation since the only alternative was morphine.

Furstenberg (1924) from the University of Michigan reported on two cases of temporal bone cancer out of 40,000 patients seen at the University of Michigan [7]. He recommended radical excision by radical mastoidectomy, employment of a technique that will allow inspection of the cavity for the remainder of the patient’s life for future cauterizations in the event of recurrence. Albert Carl Furstenberg (1890–1969) was born in Saginaw, Michigan, and graduated from the University of Michigan with a BS degree in 1913 and from the medical school in 1915 (Fig. 1.7). He remained there for residency training under Dr. Roy Bishop Canfield and for his entire professional career [29]. He was promoted to professor and chairman of Otolaryngology at the University of Michigan in 1932. In 1934, he initiated a 4-year residency program in otolaryngology, preceding by 30 years this requirement in the USA. In 1935, he was named dean of the medical school and remained in that position for 28 years [30]. Under his direction the University of Michigan Medical School grew remarkably, adding eight new buildings to the medical center and founding the Kresge Hearing Research Institute. He was a tremendous promoter of medical education and research in otolaryngology [31, 32]. His greatest contribution to otology is recognizing the amelioration of Meniere’s disease with sodium restriction [33]. He served two terms as president of the Triological Society and a term as president of the American Association of Medical Colleges (1946) and president of the American Otological Society (1953) [29]. He died in 1969 after suffering a subarachnoid hemorrhage.

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Fig. 1.7

A.C. Furstenberg (reprinted with permission) [29]

Development of Radical Mastoidectomy and Radiotherapy

In 1930 Fraser recommended radical surgery followed by radium or radiation [34]. J.S. Fraser (1874–1936) was a Scottish otologist who studied in London and Vienna (Fig. 1.8). In 1905 he became a fellow in the Royal College of Surgeons of Edinburgh. He was recognized as a world expert on temporal bone pathology, and he later became president of the Otological Section of the Royal Society of Medicine in 1927–1928 [35, 36].

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Fig. 1.8

J.S. Fraser (reprinted with permission) [35]

In 1935, Thorell in Stockholm reported on 13 patients with temporal bone cancer treated with radium [37]. Dr. Ivan Thorell wrote many papers on the therapeutic use of radium. A biography or obituary of him could not be located.

In 1943, Figi and Hempstead from the Mayo Clinic were first to report a large series of patients with middle ear and mastoid cancer including radical mastoidectomy, electrodessication, and implantation of radium [38]. Their report covers 48 patients with cancers in the middle ear and mastoid of whom 10 cases were beyond treatment, and they quote an incidence at the Mayo Clinic of 0.003% of all patients registered. This paper set the treatment for the next decade and was highly cited by contemporary authors [39]. This form of treatment always meant radiating an open mastoid cavity.

Frederick A Figi (1892–1970) was an alumnus of the University of Nebraska and was a fellow at the Mayo Clinic in 1918 and served that institution for 39 years (Fig. 1.9). He received his American Board of Otolaryngology certification in 1926. He specialized in head and neck cancer, especially laryngeal cancer, and he earned the nickname fearless Fred. He was unusually short and required specially made surgical gowns and operating room steps. During his time at the Mayo Clinic, the medical specialties were not sorted out in their current form, and he served in the section of Plastic Surgery and Laryngology. Later in life, Figi dedicated himself to plastic and reconstructive surgery. He served as the president of the American Society of Plastic and Reconstructive Surgeons and as chair of the American Board of Plastic Surgery in 1957–1958.

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Fig. 1.9

Frederick A. Figi (reprinted with permission) [88]

In a second paper, Figi writes, If it is true that here ‘fools rush in where angels fear to tread,’ it is no less true that a defeatist attitude and a halfhearted attempt at eradication on the part of the surgeon is tantamount to the death sentence for most patients [40]. In this report he recommended radical mastoidectomy, occasionally preceded by ligation of the carotid artery and electrocoagulation of tumor, followed by implantation of radium and subsequent external beam radiation. The wound was packed with iodoform gauze impregnated with petrolatum and allowed to heal by secondary intention. Unfortunately, despite these extreme and heroic efforts, he could not show a survival benefit.

Adams and Morrison in 1955 described a case series of 27 patients from the UK [41]. It must be borne in mind that these patients with middle ear and mastoid cancers were examined only with plain radiographs and that mastoidectomy was not only therapeutic but also diagnostic. They cite the method of Figi and Hempstead as the usual course of treatment. Radiotherapy was given postoperatively in all cases and consisted either of radium inserted into the operative cavity or external radium beam [41]. Their paper highlights the fact that these patients died of local disease and not regional or distant metastasis.

William Stirk Adams (1896–1976) was born in Acocks Green, Worcestershire, England, and attended Birmingham University (Fig. 1.10). His studies were interrupted by World War I, and he served as a surgeon probationer and as a boat spotter, perched high up in a balloon. Given the shortage of surgeons during the war, surgeon probationers were third- and fourth-year medical students enrolled into the navy with some basic medical training. He received his MB from Birmingham University in 1921. He began his surgical residency in obstetrics, but he switched to otolaryngology. He obtained his FRCS in 1924, and he was appointed assistant surgeon at General Hospital Birmingham in 1926. He spent his entire career in Birmingham until his retirement from the National Health Service in 1961. He was a Hunterian Professor at the Royal College of Surgeons in 1944. Although a general otolaryngologist, he demonstrated interest in otology and the Eustachian tube. He was part of a research council charged with studying the effects of radiation to the nasopharynx in cases of otitis media with effusion. In 1947, he founded the Midland Institute of Otology (MIO) for the sole purpose of postgraduate training in otolaryngology. He had many hobbies and was an accomplished gardener, bee keeper, and sailor. He drove a 1933 Rolls-Royce 20/25 four-door saloon until his death in 1971. He remained a bachelor, and upon his death, his library and the proceeds from his estate helped to fund the MIO.

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Fig. 1.10

William Stirk Adams (reprinted with permission) [89]

His colleague Robert B.I. Morrison (1913–1992) graduated from Edinburgh University in 1935. He went on to obtain his FRCP, FRCS, and MD from Edinburgh. He earned his Diploma in Radiology followed by Fellowship in FCR in 1951. In 1953, he joined the Radiotherapeutic Research Unit at Hammersmith Hospital and was responsible for the first linear accelerator for clinical use [42]. Over his long career, he saw many changes in radiotherapy. He wrote 44 papers, principally on radiotherapy techniques. He was a president of the Royal College of Physicians, Radiology section, 1977–1978. His presidential address was entitled Progress of radiotherapy and oncology from radium to platinum. [43]

Boland and Paterson described their radiotherapy technique in 1955 [44]. They reported results of radiotherapy in 14 patients with carcinoma limited to the ear canal. The overall survival rate was 23%, but the end point was not clearly defined. They described the complications of osteoradionecrosis and brainstem damage with radiation in this location. Boland published just this one paper. Ralston L. Paterson (1897–1981) published 28 papers (Fig. 1.11). He served in World War I with the Argyll and Sutherland Highlanders and received the Military Cross. He completed his medical training at the Royal Infirmary of Edinburgh in 1923. He received his training in radiotherapy at the Mayo Clinic. Along with Herbert Park, he pioneered the development of the Paterson-Parker rules for the Radium Dosage system , also called the Manchester system .

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Fig. 1.11

Ralston Paterson (https://​commons.​wikimedia.​org/​wiki/​File:​Ralston_​Paterson_​(1897–1981).png)

The earliest reported case series of sarcoma of the temporal bone is from Eggston and Wolff (1947), where they reported five cases all presenting with facial paralysis [45]. In 1952, Aub described the first case of epidermoid carcinoma of the middle ear in a radium dial painter [46]. The first case of temporal bone carcinoma from therapeutic radiation was described by Ruben et al. in 1977 [47].

Development of Surgical Techniques

Radiotherapy was the mainstay of temporal bone cancer treatment until 1951 when Ward et al. [48] described piecemeal excision of the temporal bone and surrounding structures, even if this required a radical operation, with the goal of trying to achieve negative margins. Grant Eben Ward (1896–1958) was born in Lorain, Ohio (Fig. 1.12). He graduated from Johns Hopkins Medical School in 1921. He became a fellow in the American College of Surgeons in 1928 and certified by the American Board of Surgery in 1939.

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Fig. 1.12

Grant Ward (reprinted with permission) [49]

Dr. Ward overcame significant personal medical hurdles in his career. He had ulcerative colitis and required an ileostomy in 1940. He developed a benign spinal cord tumor in 1942; and following resection, he developed severe weakness of his dominant right arm. Through perseverance and ingenuity, he worked with engineers to develop an orthotic device to support his right hand and to continue to perform surgery, developing significant dexterity with his left hand [49].

He developed an interest in electrosurgery and authored a book on the subject with Dr. Howard Kelly in 1932. He performed the first composite resection for oral cavity cancer in 1932. He wrote papers on the composite resection of oral cavity cancers and neck dissection along with radical operation of middle ear and temporal bone cancers. In 1950 he co-wrote with James Hendrick the first comprehensive textbook of head and neck surgery, Tumors of the Head and Neck. The foundation of the Society of Head and Neck Surgeons (SHNS) has its start with Drs. Hayes Martin and Grant Ward in 1953 [50]. Ward was elected to the presidency of the SHNS in 1957, but his death from rectal cancer in 1958 prevented him from serving his term [49]. Forty year later, The SHNS merged with the American Head and Neck Society in 1998 [51].

In 1951, Campbell et al. [52] suggested that en bloc resection of the temporal bone would be possible. Eldridge Houston Campbell (1901–1956) did his undergraduate work at the University of Virginia and graduated from medical school at Johns Hopkins University in 1927 (Fig. 1.13). His undergraduate honors include Phi Beta Kappa and 2 years as a Rhodes scholar. He spent his entire residency training with the eminent neurosurgeon, Dr. Walter Dandy. He joined the faculty of Albany Medical College in 1934 and served as professor and chairman of the department of surgery from 1946 until his death in 1956. During World War II, he served as chief of Surgery in the 33rd General Hospital and as consultant in Neurosurgery in the Mediterranean Theater of Operation. Given his war experiences, he wrote many papers regarding penetrating trauma to the head. He wrote the obituary of Dr. Dandy for the American Association of Neurological Surgeons [53].

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Fig. 1.13

Eldridge Houston Campbell (reprinted with permission) [90]

However, it was not until 1954 that Parsons and Lewis actually performed the first one-stage subtotal temporal bone resection in 1954 [54]. This marked a major departure from the previous treatment paradigm of radical mastoidectomy followed by radiotherapy and fostered the trend toward en bloc resection of temporal bone tumors [55]. After soft tissue surgery in the neck controls the major vessels, posterior and middle fossa craniotomy allows osteotomy across the petrous portion of the temporal bone just lateral to the internal auditory canal and removal of an intact specimen. Reconstruction was with local flaps, fascia lata, and abdominal fat grafts. The operative mortality rate was around 10% [56].

Herbert Lynn Parsons (1909–1995) was born in New York City and attended Yale University and Harvard Medical School. He did his training at St. Luke’s Hospital in New York and served during World War II in the European Theatre of Operations (ETO) as a neurosurgeon. He was an attending physician at many NYC hospitals including New York Presbyterian Hospital, Department of Neurosurgery.

John S. Lewis (1919–2005) was born in Medicine Hat, Alberta and graduated from medical school at the University of Alberta, Edmonton, in 1943 (Fig. 1.14). He served in the Royal Canadian Army Medical Corps ETO in 1943–1946 [57]. He did graduate training in surgery and pathology before completing his otolaryngology training at the University of Pennsylvania (1948–1950) and fellowship in head and neck surgery at Memorial Sloan-Kettering in 1951. He spent his entire medical career at the Memorial Sloan-Kettering Cancer Center, New York. He received numerous awards including the Newburn Medal for Surgery from the University of Alberta and the Newcomb Award from the American Laryngological Society. He served as president of the American Society of Head and Neck Surgery (1970–71) and American Otolaryngological Society (1983–84).

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Fig. 1.14

John S. Lewis (reprinted with permission) [57]

Lewis ultimately published 10 papers dedicated to cancers of the ear and temporal bone. In 1975, Lewis reported his 20-year experience with 100 cases of temporal bone cancer, 86% of which were squamous cell carcinoma. His 5-year survival rate was 25%, and the operative mortality dropped from around 10% to 5% [56]. He attributed improved results to the use of hypotensive anesthesia, high-speed drills, and adequate soft tissue coverage of the defect. His most widely cited paper, Temporal Bone Resection: Review of 100 cases [56], has lasting relevance today.

In 1960 Conley and Novak describe en bloc resection of the bony ear canal lateral to the facial nerve [58]. This description is the earliest for today’s lateral temporal bone resection (LTBR). In their series, 66% of the cases were SCC; and their 5-year survival rate was 18%. They observed that tumors that extended beyond the Eustachian tube had a very poor prognosis and that the goal of surgery should be palliation [58].

In 1965, Conley described his experience with middle ear cancers [59]. He pointed out the difficulty in curing patients when the tumor involves the Eustachian tube, petrous portion of the temporal bone, the dura, the base of skull, or extension into the neck. In the same article, he utilizes the hypoglossal-facial graft for facial paralysis. In these first articles, Conley used a chisel for lateral temporal bone resection. It was not until later that the use of high-speed drill and microsurgical dissection was described for LTBR [60].

John J. Conley (1912–1999) was born in Carnegie, PA, a small steel mill town outside of Pittsburgh (Fig. 1.15). He received his medical degree from the University of Pittsburgh. He interned at Mercy Hospital in Pittsburgh, and the nuns there advised him to study cardiology. He left Pittsburgh to do cardiology training at Kings County Hospital in Brooklyn. Ironically, during that training, he developed an arrhythmia, paroxysmal atrial tachycardia. He was counseled that cardiology was too stressful, and he should go into a field with better working hours, like ENT. Following otolaryngology training at Kings County, he served in the US Army for 4 years during World War II. The experience of treating war wounds was invaluable for his future as a head and neck surgeon [61]. After the war, he returned to NYC to work with Dr. George Pack at Memorial Hospital. He trained many residents and fellows during his long tenure at the College of Physician and Surgeons at Columbia University. He authored more than 300 papers and published 13 books in otolaryngology. He developed many innovative techniques in otolaryngology and reconstructive surgery. In addition to his scientific works, he wrote many papers on medical ethics and 11 volumes of poetry. He was fond of classical music and proficient on the flute.

../images/393691_1_En_1_Chapter/393691_1_En_1_Fig15_HTML.gif

Fig. 1.15

John Conley (reprinted with permission) [61]

He is perhaps the most famous American head and neck surgeon since Dr. Hayes Martin. He was the president of the American Academy of Ophthalmology and Otolaryngology (1974) at a time when it was being restructured to separate these two surgical specialties. He also served as president of the American Academy of Facial Plastic and Reconstructive Surgery. As the first president of the American Society of Head and Neck Surgery, he calmed the inter-specialty tension among general surgeons, plastic surgeons, and otolaryngologists. He established the John Conley Foundation for Ethics and Philosophy in Medicine. He stopped performing surgery at age 80 and retired from Columbia University’s College of Physicians and Surgeons, Columbia-Presbyterian Medical Center, and St. Vincent’s Hospital in Manhattan. As a lasting tribute, he is remembered through the John Conley lecture of the AHSN.

In another ironic twist of fate, he died from a primary tumor at the skull base, involving the facial nerve, and another malignant growth in the parotid region [61].

Hilding and Selker [62] in 1969 described a total temporal bone resection utilizing an approach first described by Julius Lempert in 1937 [63]. Lempert described an approach to the anterior petrous apex for petrous apicitis by exposing and following the carotid artery. The key step in this procedure is exposure of the carotid artery through the glenoid fossa after removal of the mandibular condyle, thus avoiding any surgery within the middle ear or temporal bone. In Hilding and Selker’s procedure , the carotid artery, jugular bulb, sigmoid sinus, and middle fossa dura are separated from the temporal bone. A curved osteotome is inserted through the posterior fossa, and sharp blows are used until the bone fractures free. The advantage of this procedure, over that from Parsons and Lewis, is the removal of the anterior portion of the temporal bone.

David A Hilding (1930–2016) was born in Duluth, Minnesota. He received his MD degree from the University of Minnesota in 1951. He was a resident at Columbia Presbyterian Hospital in New York before serving as a medical army officer during the Korean War. He spent a year conducting research at the Karolinska Institute in Stockholm before joining the faculty at Yale Medical School. His main research interest was hearing loss, and he helped to develop technology to test newborn hearing [64]. He published more than 60 articles; his most highly cited papers deal with the development of the organ of Corti and the anatomy and physiology of the onset of auditory function.

Robert G. Selker (1930–2010) grew up in Squirrel Hill neighborhood of Pittsburgh, Pa, and attended University of Pittsburgh [65]. His college career was interrupted by military service during the Korean War. After the war, he completed his undergraduate training and later his medical school at the University of Pittsburgh. He did his neurosurgical residency training at the University of Pennsylvania. He served a number of academic centers including Yale, Emery, University of Chicago, the NIH, and then Pittsburgh. He held the position of chief of neurosurgery at UPMC Montefiore. He wrote more than 100 papers, with the majority dedicated to gliomas. He developed a pioneering shunt, the Selker reservoir . He is the lead author on the influential paper regarding the Brain Tumor Cooperative Group NIH trial 87-01 [66].

In 1984, Graham et al. described the first successful, single-stage total temporal bone resection with carotid artery sacrifice [67]. This procedure built on the experience of prior surgeons. A key preliminary step to this procedure is application of a Kindt clamp to the internal carotid artery, with gradual occlusion over 4 days if tolerated by the patient. Total temporal bone resection is then performed, using fascia lata grafts for the dura and packed with abdominal fat graft. This procedure invariably produced deficits of cranial nerves VII through XII and was used successfully in a few reported cases. In 1987, Sataloff et al. reported the extended temporal bone resection, which includes the lower cranial nerves, petrous apex, carotid artery, jugular vein, sigmoid sinus, and most of the middle fossa floor [68]. However, the operative morbidity is significant without significant survival benefit, and current chemotherapy and radiotherapy techniques have largely rendered this procedure obsolete.

Malcolm D. Graham graduated medical school from McGill University in Montreal, Quebec, in 1957. He did an internship at University of Colorado Hospital in Denver, Colorado, and then returned to Canada for residency in General Surgery at Veteran’s Hospital in Victoria, BC, before completing his otolaryngology training at the University of Iowa under Dr. Dean Lierle. He did fellowship training at House Ear Clinic in Los Angeles. He became board certified in otolaryngology in 1962. He served as professor at the University of Michigan in Ann Arbor from 1978 to 2004, followed by a short time at the University of North Carolina, then Mercer University, and is currently emeritus professor at Emory University in Atlanta, Georgia. He published more than 120 papers on all areas of otology-neurotology. His most highly cited paper deals with residual and recurrent cholesteatoma. He did pioneering work on the use of homograft tympanic membranes in cases of chronic otitis media. He has served as president of the American Neurotology Society and received a Presidential Citation (2006) and Award of Merit (2008) from the American Otological Society.

Lateral temporal bone resection has undergone refinements since its first description. Crabtree et al. described several modifications such as using an endaural incision with a preauricular incision to preserve the external ear. They describe a method for dissecting and preserving the facial nerve when parotidectomy is indicated [69]. In 1990, Medina et al. proposed a classification of the types of temporal bone resection, describing four different variations of lateral temporal bone resection [70]. In 1997, Moffat et al. described an extended temporal bone resection with preservation of the internal carotid artery and piecemeal resection of the petrous apex [71].

The Impact of Diagnostic Imaging and the Development of Staging Systems

Contemporary surgeons cannot fathom operating on temporal bone cancer without the benefit of cross-sectional imaging. In the first half of the twentieth century, clinical examination and plain films (AP, Stenver’s, Laws and Owen Mayer, and Poschl views) were the main guides for otologic surgeons [72]. Polytomography became the primary radiographic diagnostic test in the 1960s [73, 74]. Polytomographs could be made at 2 mm intervals and gave some definition to the middle and inner ear structures; however, the images are blurry and rather difficult to interpret. Polytomography led to both overestimation and underestimation of disease; Goodwin and Jesse found that this modality was accurate in only 1 of 13 cases [75]. This modality was quickly replaced by computerized axial tomography in the late 1970s, which was further supplanted by magnetic resonance imaging in the 1990s.

The development of cross-sectional imaging fostered the birth of rational staging systems for temporal bone cancer. In 1980, Goodwin and Jesse divided their cases into three groups based on the location of the primary: group 1 in the cartilaginous ear canal, group 2 in the bony canal, and group 3 in the middle ear and deeper structures [75]. Absolute 5-year survival rates were 57%, 45%, and 29% in groups 1–3, respectively.

In the late 1980s, Kinney and Wood [76] and Kinney [77] were unable to develop an accurate staging system based on examination and radiographic imaging. Instead, they relied on a stepwise approach to resection of ear canal and temporal bone cancers. They categorized tumors as (1) limited to the external ear canal, (2) extension to the middle ear and TM, (3) extensive disease into the mastoid, stylomastoid foramen, skull base, and dura. They reported survival rates of 85%, 83%, and 40%, respectively, and they used radiotherapy only for those cases with positive margins on final pathology.

In 1990, Shih and Crabtree converted from polytomography to CT scan imaging. They improved on the staging system proposed by Kinney and Wood by adding preoperative CT findings and devising a three-stage system: (1) localized disease, (2) extension into the temporal bone but not beyond, and (3) tumor beyond the temporal bone into surrounding structures such as parotid, neck, skull base, and dura [73]. They identified involvement of the facial nerve as a poor prognostic factor since all patients with facial nerve involvement died of disease. They recognized that early CT had limitations with soft tissue extension. They stated that disease localized to the canal can be adequately treated with en bloc lateral temporal bone resection.

In that same year (1990), Arriaga et al. describe a staging system based on preoperative clinical findings and CT findings [78]. This paper describes the earliest version of the Pittsburgh temporal bone staging system. This system used a TNM format and has been verified as a predictor of survival outcomes, and it has become a standard for reporting results in temporal bone cancer.

Contemporary Approaches to Temporal Bone Cancer

The landmark paper by Prasad and Janecka marks a clear delineation between antecedent studies and contemporary studies [79]. They evaluated 96 publications on SCC of the temporal bone and analyzed the results of 26 papers covering 144 patients. Their findings can be distilled into five major points: (1) patients with tumors confined to the ear canal fared equally with mastoidectomy , lateral temporal bone resection (LTBR), or subtotal temporal bone resection; (2) patients with tumors that involve the middle ear fared better with subtotal temporal bone resection than with LTBR or mastoidectomy; (3) the value of surgical resection for tumors that involve the petrous apex remains unclear; (4) resection of dura does not improve survival; and (5) there is insufficient data regarding resection of brain or carotid artery. As an early form of meta-analysis, this paper advanced the study of temporal bone SCC by combining and analyzing results of prior work and decanting the salient features while pointing out the gaps in knowledge about SCC of the temporal bone. The importance of this paper is underscored by the frequency in which it has been cited by other papers (89 citations identified by www.​scopus.​com). However, as explained in many of the following chapters, ear canal tumors have much higher survival with lateral temporal bone resection than with mastoidectomy alone. The other observations continue to hold true.

Intra-arterial chemotherapy was first reported by Tucker [80] in 1965 as part of treatment for middle ear cancers. He stated that five out of six patients had good results with this type of treatment. The use of intra-arterial chemotherapy has fallen out of favor in the USA, but it is still used with success in Japan and Europe [81–86].

The Japanese have led the way in the use of chemotherapy and radiotherapy for treatment of temporal bone cancer [87]. Nakagawa et al. described a group of temporal bone cancer patients treated with combination chemotherapy and radiotherapy . The encouraging results in this paper have led others to try similar protocols for large, advanced T3 and T4 tumors. This paper marks the beginning of the modern era of temporal bone cancer.

Conclusions

A rich literature regarding temporal bone cancer has evolved over the last century. While squamous cell carcinoma is the most common tumor to involve the temporal bone, many different primary tumor types can occur within the temporal bone. These different tumor types have very different biologic behaviors, and it is important to understand the natural history of each of these tumors. Equally, many different primary locations, such as periauricular skin and parotid, can involve the temporal bone. Recognizing the similarities and differences of these primary sites from the ear canal, middle ear, and mastoid is essential.

The history of temporal bone cancer treatment reveals a refinement in understanding and techniques for management. Temporal bone cancer has been recognized from the origin of otology as a field of study. As the field of otology developed, surgical techniques to treat chronic infection were applied to temporal bone cancer. The contemporaneous development of diagnostic and therapeutic uses of radiation helped to improve on the outcomes gained by surgery alone. The improvements in temporal bone resection and the reliable use of microvascular free flaps denote the current state-of-the-art for temporal bone surgery. The development of a cogent staging system has allowed physicians to compare results and to apply appropriate treatment to disease burden. The development of multidrug chemotherapy protocols marks the dawn of a new age in temporal bone cancer.

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© Springer International Publishing AG, part of Springer Nature 2018

Paul W. Gidley and Franco DeMonte (eds.)Temporal Bone Cancerhttps://doi.org/10.1007/978-3-319-74539-8_2

2. Evaluation and Staging of Temporal Bone Tumors

Joseph T. Breen¹   and Paul W. Gidley²  

(1)

Cincinnati, OH, USA

(2)

Department of Head and Neck Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA

Joseph T. Breen (Corresponding author)

Paul W. Gidley

Keywords

Physical examinationHead and neck examinationCranial nerve examinationStagingTemporal bone cancerEar canal cancerOto-endoscopyAudiometry

Abbreviations

AJCC

American Joint Committee on Cancer

BPPV

benign paroxysmal positional vertigo

CT

computed tomography

cVEMP

cervical vestibular-evoked myogenic potentials

EAC

external auditory canal

ENG

electronystagmography

ENoG

electroneuronography

MRI

magnetic resonance imaging

oVEMP

ocular vestibular-evoked myogenic potentials

PSS

Pittsburgh staging system

SCC

squamous cell carcinoma

TM

tympanic membrane

TNM

tumor node